Hour-glass deformity of the pulmonary valve: a third type of pulmonary valve stenosis.

In the 12 years from 1975 to 1987, 55 patients had open pulmonary valve surgery for isolated congenital stenosis of the pulmonary valve. Three types of pulmonary stenosis were seen: (a) dome-shaped pulmonary stenosis (34 patients); (b) dysplastic pulmonary valves with thick cauliflower-like cusps (12 patients), and (c) hour-glass deformity of the pulmonary valve, with "bottle-shaped" sinuses (nine patients). This third type has not been described before. Preoperative identification of the valve structure is important because the choice of treatment (balloon dilatation for some dome-shaped valves and excision for dysplastic and hour-glass valves) depends on the type of stenosis.

Coarctation of the aorta associated with pulmonary valve stenosis.

A 7-year-old patient had coarctation of the aorta associated with moderate and dysplastic pulmonary valve stenosis. The child underwent corrective surgery of the coarctation. This unusual association cannot be satisfactorily explained by the theory of fetal flow patterns.

Assessment of intestinal and cardiorespiratory function in children with congenital heart disease on high-caloric formulas.

Fourteen infants with congenital heart disease were investigated for failure to thrive. Assessment of intestinal function revealed minor absorptive abnormalities (mild steatorrhea in three patients, bile salt loss in four patients), delayed gastric emptying, and abnormal triglyceride loading tests. Low caloric intake (88.3 +/- 19.3 kcal/kg/day) seemed the main reason for failure to gain weight. Weight accession and cardiorespiratory rates were monitored daily during voluntary intake, a high-caloric diet by mouth, and nasogastric tube feeding. Providing 169 +/- 29 kcal/kg/day by tube resulted in weight gain with mild and transient elevation of respiratory rate at the end of the meal and increased heart rate 90 min after the meal. This regimen is a metabolically inexpensive and efficient method of supporting weight gain in children with congenital heart disease.

Permanent cardiac pacing in congenital heart disease: a follow-up study of 20 patients.

Sinus nodal dysfunction (SND) and complete heart block (CHB) in congenital heart disease (CHD) are commonly associated with congestive heart failure, syncopal attacks, and sudden death. Permanent cardiac pacing (PCP) is required to avoid these manifestations which are frequently associated with a high rate of complications, particularly in the younger age group. Twenty patients with CHD aged 4 months to 46 years underwent pacemaker implantation. Twelve (60%) were less than 20 years of age. CHB was present in 15 patients: in 10 it developed 1 week to 11 years following surgery, in two it was congenital, and in three patients it developed spontaneously with previous conduction disturbances. SND was present in 5 patients: it was congenital in two patients and developed post-operatively in three. Seventeen patients are alive and no syncopal attacks or bradyarrhythmias were recorded 2.5 to 12.5 years following the initiation of PCP. Improvement in the cardiac output was noted in most patients with heart failure. The three patients who died had adequately functioning pacemakers. Only nine re-implantations were needed, seven of them in adult patients after closure of an atrial septal defect. Our experience indicates a favourable outcome for patients with CHD needing PCP.

Prosthetic needs and demands for services of a group of elderly people in Israel.

Prosthetic needs and demands for care were investigated in a group of the elderly, residing in Romema, Jerusalem, Israel. Of the population aged 60 and above residing in the neighborhood, 34% (n = 116) were selected at random. These were examined under standardized conditions and prosthetic status and denture requirements were recorded. The findings indicate that the prosthetic status was poor and the treatment needs extensive. In all, over 83% of the study population were edentulous, the majority in both arches. Although 86.3% of the edentulous subjects possessed dentures, over 86% of these subjects needed some form of prosthetic treatment. About 60% needed new full or partial dentures and over 25% had dentures whose fit and retention could be improved by rebasing, relining or minor adjustments.

The obstructive subaortic conus.

Eleven children are reported who had stenosis under a malposed aorta with gradients of 20 to 76 mm Hg between the right ventricle and aorta. The subaortic obstruction was caused by hypertrophy of the foreshortened infundibulum and malalignment of the infundibular septum relative to the remainder of the ventricular septum. Of these 11 patients, nine had a ventricular septal defect and seven had coarctation of the aorta. Rightward deviation of the infundibulum and aorta produced an unusually long left main coronary artery that was compressed by the stent of a bioprosthetic conduit valve in one patient. Serial cardiac catheterization studies in four patients showed progressive stenosis in each. Subaortic stenosis can develop in patients with malposition of the aorta and the frequency may be greater than 5% since milder forms are likely to occur. The obstruction can be progressive. The left coronary artery may be particularly vulnerable to compression after operative repair with an extracardiac conduit.

Retroesophageal right aortic arch.

Right aortic arch with left descending aorta and a retroesophageal aortic segment is an uncommon congenital vascular malformation which may cause symptoms of compression from a vascular "ring." The presence of this malformation may be suspected by the findings on barium swallow and the diagnosis confirmed by angiocardiography. Surgical intervention may be necessary in patients who have severe symptoms.

Two-dimensional echocardiography in discrete subaortic stenosis.

Thirty-seven patients with discrete subaortic stenosis (DSS) underwent 2-dimensional echocardiography (2-D echo) and cardiac catheterization. The peak systolic pressure gradients ranged from 0 to 150 mm Hg. Thirty-two patients had membranous DSS and 5 had fibromuscular DSS. Of 37 patients with DSS, 2-D echo diagnosed the presence and type in 35; in 2, a membrane was demonstrated by angiography. Of the 35 patients accurately diagnosed by 2-D echo, angiography corroborated the diagnosis in 33, but failed to show the membrane in 2. Subsequent cardiac surgery confirmed the accuracy of the echocardiographic diagnosis in these 2 patients. In all patients with membranous DSS, the anterior insertion of the membrane was demonstrated. In 9 of them the posterior insertion was demonstrated by tilt of the transducer but the anterior insertion disappeared. In 4 patients both insertions were demonstrated simultaneously and in 3 patients the membrane was demonstrated as a continuous line. In 4 of the 5 patients with fibromuscular DSS, both insertions of the lesion were demonstrated simultaneously. However, 2-D echo was unsuccessful in assessing the severity of obstruction. In only 1 patient did demonstration of the whole subaortic membrane as a continuous line below the aortic valve correlate with severe obstruction. Thus, the presence and type of DSS, but not the degree and severity, can be accurately and reliably diagnosed by means of 2-D echo.

The renal effects of radiocontrast administration during cardioangiography in two different groups with congenital heart disease.

Renal effects of the administration of contrast media during cardiac catheterisation were compared in two groups of patients with congenital heart diseases. Group A consisted of 21 patients with cardiac malformations, characterised primarily by left ventricular volume overload and known to be associated occasionally with left heart failure, such as: left to right shunts and left ventricular valvular defects. Group B consisted of 23 patients with lesions affecting the right ventricle which are rarely associated with left heart failure, such as: Tetralogy of Fallot and Pulmonic stenosis. Patients in Group A showed a significant increment in both plasma creatinine and uric acid levels in the 24 h following heart catheterisation. This observation was significantly more prominent in the older age group (above the age of 5 years). In Group B no changes in these parameters were encountered. Plasma renin activity and fractional sodium excretion increased and decreased respectively, by a similar degree in both groups in the 24 h following contrast media administration. No difference in renal tubular handling of uric acid was observed between both groups, nor did any of the patients studied demonstrate any degree of proteinuria or abnormality in the urine sediment, prior to or following heart catheterisation. We suggest that chronic pre-existing left ventricular overload should be considered a risk factor among the other known risk factors which promote the incidence of acute renal failure after contrast media administration.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis of pulmonic regurgitation by contrast echocardiography.

In order to determine whether pulmonic regurgitation (PR) can be reliably diagnosed using contrast echocardiography, we studied 24 subjects using echocardiography during intravenous injections of 5% dextrose solution. Twelve were without PR, and twelve had PR (10 after intracardiac repair of tetralogy of Fallot, one after a Brock procedure for pulmonic stenosis, and one after insertion of a right ventricle-pulmonary artery conduit for pseudo truncus arteriosus). Two blinded independent observers correctly diagnosed PR in all patients when it was present, and correctly excluded it in 11 of 12 of the patients without PR. They disagreed in one patient who had an unusual contrast pattern during diastole. Four postoperative patients had PR by contrast echocardiography despite the absence of a cardiac murmur at the time of their study. We conclude that contrast echocardiography is a safe, simple, sensitive, and specific method for diagnosing PR. Some methodologic suggestions and limitations of the method are discussed.

Persistent left ventricular disease in clinically "cured" primary endocardial fibroelastosis.

We studied by serial cardiac catheterisation eight patients with the dilated form of primary endocardial fibroelastosis in whom congestive heart failure disappeared with treatment. All remained without symptoms for at least three years before recatheterization. Four patients showed regression of the abnormal electrocardiographic findings, three showed persistence, and one showed progression of electrocardiographic left ventricular overload pattern. On first cardiac catheterisation all patients had a dilated left ventricle with a mean ejection fraction of 0.36. In six of the patients repeat cardiac catheterisation showed left ventricular dilatation with a diminished ejection fraction (mean 0.32). Left ventricular end-diastole pressure was raised (12 to 28 mmHg, mean 19 mmHg). In this group were included the three patients with persistence and one with progression of the abnormal electrocardiographic findings, and two of the four patients with regression of these findings. The highest left ventricular end-diastolic pressure was found in a patient in whom the abnormal electrocardiographic findings almost reverted to normal. In the two remaining patients with reversion of the electrocardiographic abnormalities repeat cardiac catheterisation showed nothing abnormal. Our findings indicate that "cure" in primary endocardial fibroelastosis is incomplete. These findings may be the cause of sudden death or late clinical deterioration in some reported patients with "cured" primary endocardial fibroelastosis. The electrocardiogram is of little value in assessing these processes.

Discrete subaortic stenosis associated with congenital valvular aortic stenosis--a diagnostic challenge.

Discrete subaortic stenosis has only rarely been reported in association with congenital valvular aortic stenosis. The valvular lesion may obscure the subaortic stenosis or may be obscured by it. Our experience with seven such cases (10% of our 71 patients with discrete subaortic stenosis) is reported and the diagnostic problems are discussed. In six cases the discrete subaortic stenosis was membranous and in one it was a fibromuscular tunnel. Two patients had associated coarctation of the aorta, two had aortic insufficiency, and one had a ventricular septal defect. An important clinical clue to the diagnosis was an ejection systolic click, which was found in four patients. Four patients were accurately diagnosed at cardiac catheterization. Two patients, in whom cross-sectional echocardiography (2DE) was performed, were accurately diagnosed by it.